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Aims: We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) as information concerning the natural history and risk stratification of ARVC is still incomplete., Methods and Results: A cohort of 96 ARVC patients (68% males, 35 ± 15 years) was enrolled and underwent structured diagnostic protocol and follow-up. Primary study endpoints were death and heart transplantation (HTx). Clinical and echo-Doppler data were assessed as prognostic indicators. Sixty-five per cent of patients had right ventricular (RV) systolic dysfunction (RV fractional area change < 33%) and 24% had left ventricular (LV) systolic dysfunction (LV ejection fraction <50%). During a mean follow-up of 128 ± 92 months, 20 patients (21%) experienced cardiac death or underwent HTx. At multivariate analysis (Model 1), RV dysfunction [hazard ratio (HR): 4.12; 95% confidence interval (CI): 1.01-18.0; P = 0.05], significant tricuspid regurgitation (HR: 7.6; 95% CI: 2.6-22.0; P < 0.001), and amiodarone treatment (HR: 3.4; 95% CI: 1.3-8.8; P = 0.01) resulted as predictors of death/HTx. When inserting in the model, the 'ordinal dysfunction' (Model 2), which considers the presence of both RV and LV dysfunctions, this variable emerged as an independent prognostic predictor (HR: 6.3; 95% CI: 2.17-17.45; P < 0.001). At the receiver operating characteristic analysis, Model 2 was significantly more accurate in predicting long-term outcome compared with Model 1 (area under the curve 0.84 vs. 0.78, respectively; P = 0.04)., Conclusion: In our tertiary referral centre ARVC population, the presence of LV dysfunction at diagnosis has an incremental power in predicting adverse outcome compared with RV dysfunction alone.

In a prospective study on 340 cases with primary myocardial disease, eight patients (six males, two females, mean age 36 years, range 24-47) with an idiopathic left ventricular aneurysm were observed. All patients had normal coronary arteries, no angina or history of myocardial infarction; all but one had no risk factors for ischaemic heart disease; all had normal right ventricles; one patient had a history of familial dilated cardiomyopathy, two of 'flu-like' syndrome at the time of first symptom and two of alcohol abuse. All patients had ventricular tachycardia (VT), five sustained (of right bundle branch block morphology in three, and of different morphologies in two), three non-sustained. Patients with sustained VT had inducible VT (resembling the clinical one) on electrophysiological study. Electrocardiogram (ECG) showed an infarction pattern in three cases. Aneurysms were of limited size (2.1 +/- 1/11 segments on echocardiography) and were located in the septum, apex or posterior wall. Left ventricular ejection fraction (LVEF) was reduced (< 0.50) in six patients and was not correlated with the aneurysm size. The duration of illness was inversely correlated with LVEF (P < 0.05). Endomyocardial biopsy showed evidence of diffuse pathological changes in all cases (cell hypertrophy, myofibrillar lysis, mitochondriosis). During follow-up (64 +/- 32 months), patients were successfully treated with anti-arrhythmic drugs: no patients required surgical treatment to control ventricular arrhythmia. Considering the clinical and pathological features of idiopathic left ventricular aneurysm, this primary myocardial disease could be classified as a novel peculiar form of cardiomyopathy.

Right ventricular cardiomyopathy is generally considered a disease with isolated right ventricular involvement, although there are some case reports of concomitant abnormalities of the left ventricle. In order to evaluate the prevalence, characteristics and natural history of left ventricular involvement in this disease, 39 patients with right ventricular cardiomyopathy were studied by 2-dimensional echocardiography. At first evaluation 25 patients (group 1) showed right ventricular abnormalities and a normal left ventricle. In the remaining 14 patients (group 2) the right ventricular abnormalities were associated with left ventricular involvement (asynergic areas and/or mild diffuse hypokinesia). During the follow-up, left ventricular involvement appeared in 6 patients of group 1 and worsened in 4 patients of group 2. Five patients died. Pathological examination (available in 4 cases) showed, at the level of the right ventricle, marked atrophy of myocytes and fibro-fatty replacement, and predominant fibrosis at left ventricular level. Left ventricular involvement is thus frequent in right ventricular cardiomyopathy. At least in some cases the disease appears to be progressive.

While coronary reperfusion arrhythmias are well defined in animal models, their prevalence and predisposing factors are still uncertain during pharmacologic coronary reperfusion in patients with acute myocardial infarction. In 80 patients, who underwent this procedure and had a continuous electrocardiographic monitoring (associated with a Holter recording in 34), 6 patients developed ventricular fibrillation. Ventricular fibrillation was the terminal event in 2/19 non-reperfused patients. The other episodes of ventricular fibrillation (controlled with DC countershock) occurred within 5 minutes and 3 minutes after angiographic documentation of coronary reperfusion. Ventricular fibrillation was observed in 4/10 patients with a residual stenosis less than or equal to 80% but in no case among the 39 patients with a residual stenosis greater than 80% (P = 0.01). Holter recordings in 19 reperfused patients showed ventricular tachycardia or accelerated idioventricular rhythm at reperfusion in 8/10 patients with a coronary stenosis less than or equal to 80% and only in 3/9 with a higher degree stenosis (P less than 0.05). Reperfusion ventricular fibrillation was found to occur in 8.1% of patients. A residual stenosis of 80% or less was found to be a predisposing factor in the occurrence of ventricular fibrillation, ventricular tachycardia or accelerated idioventricular rhythm at reperfusion.

In order to investigate the anatomic substrate of 'idiopathic' ventricular tachycardia (VT) 10 patients with chronic recurrent VT and no apparent sign of heart disease underwent an echocardiographic, haemodynamic and histologic study (5 males, 5 females: mean age = 40 +/- 11 years). In the patients with a left bundle branch block morphology of VT (7 cases), four showed findings compatible with an arrhythmogenic right ventricular dysplasia or a right ventricular cardiomyopathy. In the other three all examinations were normal with the exception of endomyocardial biopsy, which showed slight non specific changes in two. Of the remaining 3 cases (characterized by a right bundle branch block morphology of VT or by the presence of polymorphic VT) one had histologic evidence of myocarditis while another developed dilated cardiomyopathy. Macroscopic and/or microscopic ventricular abnormalities are frequently found in patients with VT which appears idiopathic. In these cases myocardial disease is frequently progressive, despite optimal control of VT.

To determine the antiarrhythmic efficacy of beta-blockers (beta-B) and verapamil (V) in exercise-induced ventricular tachycardia (Ex-VT), nine patients with reproducible Ex-VT (in two consecutive exercise tests) were studied by means of electrophysiologic study (EPS) in basal conditions and serial exercise testing after beta-B (metoprolol 25 mg tid to 100 mg qid; oxprenolol 40 mg tid) and/or V (80-160 mg tid). Ejection fraction was normal in four cases and depressed in five. Of these nine patients, four developed Ex-VT during chronic amiodarone treatment, which was continued. During EPS, VT was induced at a critical atrial pacing rate in one case, and with the extrastimulus technique in four. Ventricular tachycardia was not inducible with either technique in four patients. Five of the six patients on beta-B and none of the seven on V developed Ex-VT, although they achieved the same or higher work-loads as compared to the basal exercise tests. In the case with rate-dependent VT, beta-B and V prevented VT at work-loads, sinus rates and double products significantly higher than those obtained in basal conditions. In the others, maximal heart rate and double product were lower on beta-B and showed a wide variability on V. V and beta-B appeared to be highly effective in preventing Ex-VT, in patients with normal heart as well as in those with greatly depressed ejection fraction. Both of the drugs appeared to suppress re-entry or triggered activity in the patient with rate-dependent Ex-VT.(ABSTRACT TRUNCATED AT 250 WORDS)